Pathophysiological and diagnostic implications of cortical dysfunction in ALS

被引:0
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作者
Nimeshan Geevasinga
Parvathi Menon
P. Hande Özdinler
Matthew C. Kiernan
Steve Vucic
机构
[1] Western Clinical School,Davee Department of Neurology and Clinical Neurological Sciences
[2] University of Sydney,undefined
[3] Westmead Hospital,undefined
[4] Northwestern University,undefined
[5] Feinberg School of Medicine,undefined
[6] Brain and Mind Research Institute,undefined
[7] University of Sydney,undefined
来源
Nature Reviews Neurology | 2016年 / 12卷
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摘要
Cortical hyperexcitability is an early pathophysiological feature of amyotrophic lateral sclerosis (ALS)Cortical hyperexcitability potentially mediates motor neuron degeneration in ALS via a dying-forward, trans-synaptic, glutaminergic mechanismThe identification of the C9orf72 hexanucleotide repeat expansion as a major cause of familial and apparently sporadic ALS, as well as frontotemporal dementia, underscored the importance of cortical dysfunctionCortical hyperexcitability is an important diagnostic biomarker of ALS, and could enable more-definitive diagnosis at an earlier stage of the disease process
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页码:651 / 661
页数:10
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